RUBCN

Chr 3AR

rubicon autophagy regulator

Also known as: KIAA0226, RUBICON, SCAR15

The encoded protein negatively regulates autophagy by inhibiting PIK3C3 activity and controls endosome maturation and degradative endocytic trafficking. Mutations cause spinocerebellar ataxia, autosomal recessive 15, which primarily affects cerebellar function and coordination. This follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 0.791 OMIM phenotype
Clinical SummaryRUBCN
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
💊
Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.79LOEUF
pLI 0.000
Z-score 2.78
OE 0.58 (0.430.79)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.47Z-score
OE missense 0.83 (0.770.89)
472 obs / 570.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.58 (0.430.79)
00.351.4
Missense OE0.83 (0.770.89)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 29 / 50.3Missense obs/exp: 472 / 570.7Syn Z: 0.98
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedRUBCN-related syndromic intellectual disability with ataxia, dysarthria and epilepsyOTHERAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6552th %ile
GOF
0.5562th %ile
LOF
0.3842th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RUBCN · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →